左室双出口1例报告

左室双出口是一种极罕见的先天性心脏病 ,迄今国内文献尚未见有过报道。现将我院经心血管造影和手术证实的 1例报告如下 :患儿 ,女 ,4岁半。自幼发现心脏杂音及紫绀 ,活动后蹲踞。体检 :口唇紫绀 ,四肢杵状指 (趾 ) ,胸骨左缘第 3、4肋间闻及Ⅲ级收缩期杂音 ,Ｐ2 减弱。心电图示 :右房、右室肥大。二维超声心动图显示 :右房、室增大 ,膜部室缺 1.0ｃｍ ,主动脉骑跨35 % ,右室流出道不窄 ,肺动脉瓣显示不清 ,肺动脉及左、右肺动脉干均明显狭窄。超声诊断法乐四联症。心脏平片示双侧肺血减少 ,“靴型心” ,主动脉结增宽 ,心腰凹陷 ,心尖上抬 ,…     

超声心动图诊断法洛四联症-合并左肺动脉缺如

目的:探讨超声心动图诊断法洛四联症合并左肺动脉缺如的价值。材料和方法:分析5例法洛四联症合并左肺动脉缺如患儿超声心动图二维图像、彩色多普勒声像图表现并与心血管造影、手术结果对比分析,总结其诊断要点。结果:5例法洛四联症合并左肺动脉缺如患儿心内畸形与法洛四联症相同,主肺动脉均狭窄,4例肺动脉分叉处呈圆弧形改变,1例左肺动脉显示不清。超声诊断均与心血管造影、手术结果符合。结论:超声心动图诊断法洛四联症合并左肺动脉缺如具有较高的准确性,如患儿肺动脉分叉处呈圆弧形改变或多切面不能清楚显示左肺动脉,应高度怀疑合并左肺动脉缺如。     

小儿法乐氏四联症不同部位及特殊角度电影心血管造影（附162例分析）

近年来，随着小儿法乐氏四联症（简称法四）根治术的进展，为显示有无多发性室缺，左右肺动脉起始部狭窄及冠状动脉异常等解剖情况对手术十分重要，我院法四术前心血管造影从正侧位右室造影转而常规采用轴位成角投照右室造影及左室造影，并根据病情需要对部分病例进行了主动脉造影，现简介如下。     

超声心动图诊断右室双出口的价值和鉴别诊断

目的探讨二维超声心动图和彩色多普勒血流显像诊断右室双出口的价值。材料和方法超声检查右室双出口45例,男28例,女17例。所有病例均与手术或心血管造影对照。结果45例右室双出口超声心动图诊断符合率95．6％(43／45),2例误诊为法乐氏四联症。超声分型的符合率97．7％(42／43)。合并畸形以肺动脉狭窄最多见,占71．1％(32／45)。室间隔缺损口径平均值(21．8mm)略大于主动脉环部平均内径(2lmm)。结论二维超声和彩色多普勒血流显像相结合的检查方法对右室双出口诊断、分型和合并畸形检出具有重要价值。     

Fallot四联症的MRI诊断

笔者分析了２１例经心血管造影和手术证实的Ｆａｌｌｏｔ四联症的ＭＲＩ，探讨ＭＲＩ在显示四联症病理解剖中的作用。提出了显示不同解剖结构应采用的扫描方法，认为ＭＲＩ可以较清晰地显示四联症的肺动脉狭窄、室间隔缺损、主动脉骑跨和右心室肥厚。与其他检查方法相比，ＭＲＩ的诊断能力等于或优于超声心动图；在确定室间隔缺损类型、大小，观察右心室肥厚及肺动脉闭锁时观察中央肺动脉方面优于心血管造影检查。     

16层螺旋CT在法洛四联症诊断中的价值

目的探讨16层螺旋CT血管成像对法洛四联症的CT表现及其诊断应用价值。方法回顾性分析6例经16层螺旋CT对比增强检查的法洛四联症患者结果,6例均有手术结果及超声心动图结果对照。结果16层螺旋CT能清晰显示室间隔缺损、肺动脉或右室流出道狭窄、主动脉骑跨、右心室肥大,诊断符合率为100%;在诊断室间隔缺损程度、主动脉骑跨率方面与超声心动图无明显差异,在诊断肺动脉或右室流出道狭窄程度方面明显优于超声心动图。结论16层螺旋CT与超声心动图检查相结合,可大大提高对法洛四联症的诊断准确率。     

法乐氏四联症肺动脉分支发育不良（附2例报告）

在法乐氏四联症外科治疗的术式选择上有根治术和姑息术，随技术发展，根治术的适应症范围越来越扩大，但有无禁忌症呢？近２年来在国内外有些争论。我们将２例法乐氏四联症肺动脉分支发育不良的手术治疗作简要报告。例１：男性，５岁，生后即发现青紫，喜蹲踞，活动后心慌气短。查体：唇紫，胸骨左缘３、４肋间有３／６级收缩期杂音，杵状指（趾）。ＥＣＧ：右室肥厚。胸片：肺血少，靴型心。心脏超声：嵴下型室缺１９ｍｍ，主动脉骑跨３５％，主肺动脉７ｍｍ，左肺动脉７ｍｍ，右肺动脉７ｍｍ，右室造影：嵴下型室缺，肺动脉瓣狭窄，左右肺…     

产前彩色多普勒超声检查法洛氏四联症的临床分析

目的 探讨超声四种切面法对产前诊断法洛氏四联症的临床价值.方法 选择2010年1月～2012年12月间于我院行产前检查的法洛氏四联症患者16例,均接受超声检查,心脏检查切面选择四腔心脏切面（4CV）、主动脉根部短轴切面（AR-SAV）、左右心室流出道切面（VOTV）及三血管平面（3VV）.分析法洛氏四联症超声学图像特点,比较不同切面对法洛氏四联症诊断情况.结果 四腔心脏切面（4CV）联合主动脉根部短轴切面（AR-SAV）对胎儿法洛氏四联症诊断率为62.5.00％.而四腔心脏切面（4CV）联合左右心室流出道切面（VOTV）及四腔心脏切面（4CV）联合三血管平面（3VV）和左右心室流出道切面（VOTV）对胎儿法洛氏四联症诊断率分别为75.00％和93.75％,均显著高于四腔心脏切面（4CV）联合主动脉根部短轴切面（AR-SAV）对胎儿法洛氏四联症,差异具有统计学意义（P＜0.05）.结论 不同超声切面对法洛氏四联症诊断符合率不同,其中四腔心脏切面（4CV）联合三血管平面（3VV）和左右心室流出道切面（VOTV）对胎儿法洛氏四联症诊断率最高.     

法鲁氏四联症的肺动脉解剖

作者分析了200例四联症患者。均行右室右前斜和左前斜位造影。肺动脉狭窄的部位按Gay氏法分类如下:Ⅰ型:肺动脉干,右肺或左肺动脉狭窄;Ⅱ型:(分叉处狭窄)肺动脉干狭窄累及到左肺或右肺动脉;Ⅲ型:多发性外周肺动脉狭窄;Ⅳ型:肺动脉主干和周围肺动脉狭窄。按肺动脉狭窄的形态分为:不连续型,阶段型、弥漫型。按狭窄的程度分为:轻、中、重。依照Partide等的肺动脉狭窄处直径与同一水平降主动脉管径的比值分类为:正     

先天性右室流出道狭窄及其合并畸形的MRI

目的：研究MRI诊断小儿先天性右室流出道狭窄及其合并畸形的可靠性及限度。材料和方法：回顾分析15例小儿先天性右室流出道狭窄及其合并畸形的MRI表现，与二维超声心动图．心血管造影和手术对照比较．结果：MRI定性诊断的正确率与心血管造影一致。对其合并畸形的显示，MRI和二维超声心动图各有优缺点，而心血管造影则仍为最佳显示方法．结论：MRI对小儿先天性右室流出道狭窄的定性确诊率为100％，对合并畸形——体肺侧支，冠状动脉的显示随着MRI设备(硬件和软件)的改进，可望进一步提高而替代超声和心血管造影．     

胎儿法洛四联症的产前超声诊断

目的：探讨胎儿法洛四联症的产前超声特征。方法：对12例经胎儿超声检查诊断为法洛四联症的超声资料进行回顾性分析,总结胎儿法洛四联症的声像图特点。结果：产前共诊断12例法洛四联症。胎儿法洛四联症的主要声像图特征是室间隔缺损、主动脉骑跨和肺动脉主动脉内径比异常。结论：超声心动图产前诊断胎儿法洛四联症具有重要的临床意义。胎儿典型法洛四联症的主要声像图特征是室间隔缺损、主动脉骑跨和肺动脉内径较主动脉内径窄,右室前壁增厚不明显。     

胎儿法洛四联症的产前超声筛查及诊断

目的探讨彩色多普勒超声检查在产前诊断胎儿法洛四联症的价值和技巧。方法2010—2012年,在我院就诊待产,经产后证实为法洛四联症患儿8例,其中4例胎儿经尸解证实,4例产后超声心动图证实(2例已行手术治疗,2例随访中);采用四腔心+左、右流出道+三血管切面筛查心脏大血管,分析总结胎儿法洛四联症声图像特点。结果除1例漏诊外,其余7例产前超声筛查均作出正确诊断;法洛四联症胎儿期声图像特征主要表现为室间隔缺损、主动脉骑跨、肺动脉狭窄,右室壁增厚不明显,轻型法洛四联症容易漏诊。结论采用四腔心+左、右流出道+三血管切面筛查能减少胎儿法洛四联症漏诊,提高产前诊断率。     

肺动脉吊带的彩色多普勒超声心动图诊断

目的:探讨彩色多普勒超声心动图对肺动脉吊带的诊断价值。方法:经心导管及手术证实为肺动脉吊带的6例患者为研究对象,回顾性分析其超声心动图表现。结果:6例肺动脉吊带患者中,超声诊断3例,诊断正确率50%;漏诊3例,漏诊率50%。6例肺动脉吊带患者中,2例合并四联症,2例合并房间隔缺损,1例合并室间隔缺损,1例合并左心发育不育综合症。结论:肺动脉吊带是极为少见的先天性心脏病,常规超声心动图检查时,在正常左肺动脉起源处若未发现左肺动脉,提示可能存在肺动脉吊带。     

Magnetic resonance tomography of Fallot's tetralogy

We present four patients with tetralogy of Fallot, imaged with magnetic resonance. MRI is useful in clearly depicting the dilated ascending aorta, the infundibular stenosis, ventricular septal defect, right ventricular hypertrophy and dilated bronchial arteries. The pulmonary outflow tract can be visualized better with MR imaging than with ultrasound.     

Tetralogy of Fallot: MR findings

Surgical treatment of patients with tetralogy of Fallot requires accurate definition of all anatomic structures, particularly the central pulmonary arteries. Magnetic resonance (MR) images of 22 patients with tetralogy of Fallot were studied to assess their usefulness in providing information regarding the spectrum of anatomic abnormalities in this condition. MR findings were compared with information obtained at catheterization (in 16 patients) and at surgery (in nine patients), both of which were performed within 3 months of MR imaging. Ventricular chamber enlargement and wall hypertrophy were clearly delineated in most of the 17 patients who were examined before definitive surgical repair, and ventricular septal defects were visualized in all 17. Palliative systemic-to-pulmonary shunts were visualized in 11 patients and could be evaluated for patency. Most important, the morphology and size of the right ventricular outflow tract and central pulmonary arteries could be accurately assessed. Pulmonary artery measurements obtained from MR images demonstrated excellent correlation with angiographic measurements. In six patients examined after complete surgical repair, MR images accurately reflected changes in pulmonary artery outflow tract morphology and complications, such as residual pulmonary artery stenosis and thrombosis. The findings suggest that MR imaging can complement or obviate catheterization in the evaluation of tetralogy of Fallot with regard to suitability for definitive surgical repair.     

Congential extracardiac shunts with tetralogy of fallot

Congenital extracardiac shunts in tetralogy of Fallot may significantly alter the history and clinical findings and also have significant management implications. An unusual variant of severe tetralogy of Fallot in a 54-year-old man enabled long survival with minimal symptoms. The patient had pulmonary valvular and infundibular stenosis, a large ventricular septal defect, an overriding aorta, and large volume systemic-to-pulmonary artery shunting from his right internal mammary and accessory internal mammary arteries to his right pulmonary artery.     

婴幼儿法乐四联症的MRI诊断(附30例分析)

目的 探讨先正确性一心脏病婴幼儿法乐四联症的ＭＲＩ诊断价值。材料与方法 分析３０例婴幼儿法乐四联症ＭＲＩ所见,并与心血管造影和超声心动图比较。结果 ３０例室间隔及其缺损均在横断面扫描显示最佳,２５例主动脉骑跨在冠状面扫描显示清晰,５例显示不清。３０例肺动脉主干及右肺动脉则在矢状面扫描较清楚,其中４例还显示出第三心室。结论 ＭＲＩ检查安全、无创,优于心血管造影;能对各房室连接及心肌厚度多方位、多手段     

Pre-operative evaluation with MR in tetralogy of fallot and pulmonary atresia with ventricular septal defect

Purpose: To assess whether MR imaging could replace angiography in pre-operative evaluation of patients with tetralogy of Fallot and pulmonary atresia with ventricular septal defect (VSD), especially since the surgical correction was done earlier than was previously the rule.Material and Methods: Fourteen patients with tetralogy of Fallot (n=10) or pulmonary atresia with VSD (n=4), mean age 7.5±4.4 months, were evaluated with angiocardiography and MR before definitive surgical correction.Results: There was good diagnostic agreement between the two modalities when evaluating right ventricular outflow obstruction; 86% for valvular and 93% for supravalvular stenosis, but the agreement was somewhat lower for the subvalvular obstruction (57%). Surgery findings, however, were in favour of MR in 5 patients concerning the subvalvular right ventricular outflow tract obstruction. MR images identified all stenoses in the right and left pulmonary arteries, but overlooked one stenosis in the main pulmonary artery. MR could evaluate patency in all palliative shunts.Conclusion: Even in this young age group, MR imaging offers a good alternative to angiocardiography for the pre-operative evaluation of the right ventricular outflow tract, the main pulmonary artery and the proximal right and left pulmonary arteries, before definitive surgical correction of tetralogy of Fallot and pulmonary atresia with VSD.